Renal regenerative capacity related to stem cell reserve in nephrectomized rats.

PURPOSE: On the new era of stem cell therapy, the present experimental study was conducted to investigate renal regenerative capacity related to kidney stem cell reserve in different nephrectomy (Nx) models. METHODS: Three- and eight-week-old rats (n = 168) were randomly divided into four groups to include control and three Nx subgroups (1/6 Nx, 1/2 Nx, and 5/6 Nx) (Fig. 1). On post-Nx days 15, 30 and 60, kidney specimens were obtained to determine renal regenerative capacity. The specimens were examined with immunofluorescence. CD90/CD105 and Ki-67 expressions were determined as stem cell and cellular proliferation markers, respectively. Fig. 1 Intraoperative photographs showing three different types of nephrectomies (unilateral total Nx has not been shown in 5/6 Nx group) RESULTS: CD90 and CD105 expressions were stronger in glomeruli, but Ki-67 expressions were present only in tubuli. When all Nx types and post-Nx days were considered, both 3- and 8-week-old rats undergone 5/6 Nx had the highest glomerular CD90 and CD105 double expressions. While the expressions gradually increased toward the day 60 in 3-weeks old rats, 8-week-old rats had almost stable double expressions. The strongest tubular Ki-67 expressions were seen in 5/6 Nx groups of both in 3- and 8-week-old rats. The expressions were strongest on day 15 and then gradually decreased. Ipsilateral 1/6 Nx groups had stronger Ki-67 expression than contralateral ones in both age groups. CONCLUSIONS: Kidneys may pose a regenerative response to tissue/volume loss through its own CD90- and CD105-related stem cell reserve which mainly takes place in glomeruli and seems to have some interactions with Ki-67-related tubular proliferative process. This response supports that kidney stem cells may have a potential to overcome tissue/volume loss-related damage.

Epimorphin expression in a rat model of pulmonary hypoplasia associated with congenital diaphragmatic hernia.

PURPOSE: The pathogenesis of pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) remains unclear. Interactions between the epithelium and surrounding mesenchyme play an important role in normal lung morphogenesis. Epimorphin, a stromal protein, plays a role in epithelial morphogenesis and lung branching, both of which are involved in pulmonary hypoplasia. In this study, we aimed to examine the relationship between epimorphin and pulmonary hypoplasia associated with CDH in an animal model. METHODS: Time-pregnant rats were exposed to nitrofen or vehicle on gestational day 9 (D9). Fetuses were harvested on D16 and D20, and were divided into control, hypoplastic lungs with CDH (CDH+), and hypoplastic lungs without CDH (CDH-). Both lungs of each fetus were removed and subjected to morphometric and molecular biologic analyses. Lung-to-body weight ratios were calculated. Pulmonary RNA was extracted, and relative mRNA level of epimorphin was determined by quantitative real-time PCR (qRT-PCR). Protein expression of epimorphin was investigated by Western blotting. RESULTS: In groups D16 and D20, lung-to-body weight ratios in subgroups CDH+ were significantly lower than those of controls and CDH-. The relative mRNA expression levels of epimorphin were significantly increased in both lungs in subgroup CDH+ compared with controls and CDH- on D16. Pulmonary epimorphin gene expression levels were significantly decreased in CDH+ group on D20 compared to controls. Western blotting confirmed the qRT-PCR results showing decreased pulmonary epimorphin protein expression in CDH+ hypoplastic lungs compared to controls on D20. CONCLUSION: Our study shows that there is an association between the epimorphin expression and pulmonary hypoplasia associated with CDH. Although the cause-effect relationship is far from being established, epimorphin-related mechanisms have a more critical role in early (D16) developmental stage.

Multidisciplinary Management of Pediatric Hepatoblastoma: A 20-Year Single-Center Experience.

BACKGROUND: Hepatoblastoma is rare cancer that responds well to risk-based chemotherapy, and surgical treatment is needed to achieve complete remission and satisfactory survival rates in hepatoblastoma patients. In this study, we evaluated the clinical features and treatment outcomes of pediatric hepatoblastoma patients treated in our clinic. METHODS: Eighteen patients with hepatoblastoma who were treated and followed up in our center between June 1999 and June 2020 were analyzed retrospectively. All patients were evaluated by a multidisciplinary team and managed using a risk-based protocol (SIOPEL-1 and SIOPEL-3). RESULTS: The patients' mean age at diagnosis was 38.33 ± 52.34 months. Sixteen patients (89%) received neoadjuvant chemotherapy, and 2 patients (11%) who underwent complete mass excision at diagnosis received adjuvant chemotherapy. After neoadjuvant therapy, the tumor was completely resected in 8 patients (45%), while liver transplantation was performed in 6 patients (34%) because complete resection of the tumor was not possible. Two patients died before surgical treatment. One patient relapsed with lung metastasis after salvage chemotherapy. She is alive without disease at 64 months. The mean follow-up time was 59.3 ± 49.8 months; 5-year overall and disease-free survival rates were 88.9% and 80.8%, respectively. The 5-year overall survival rate was 100% for both liver transplant and resected patients, whereas 5-year disease-free survival was lower in transplant patients (75% vs 100%, P < .001). CONCLUSION: Multidisciplinary follow-up is especially important for patients who may need liver transplantation. Some patients may benefit from new treatment options such as radiofrequency ablation and cyberknife treatment.

Treatment of Wilms tumor: a report from the Turkish Pediatric Oncology Group (TPOG).

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Does massive proximal small bowel resection influence prostaglandin E2 synthesis in the stomach and ileum during adaptive process in rats?

It is unclear whether massive small bowel resection (SBR) affects prostaglandin E2 synthesis in the gastrointestinal tracts. Thus the aim of this study was to investigate possible changes over tissue levels of prostaglandin E2 in the stomach and ileum after massive proximal SBR. Female Swiss-Albino rats underwent control operation (groups 1, 3, 5) or an 80% SBR (groups 2, 4, 6). The specimens were obtained during relaparotomy at 3 days in groups 1 and 2, at 9 days in groups 3 and 4, at 15 days in groups 5 and 6. Group 2 vs. groups 1 and 6, group 4 vs. groups 3 and 6 had significant increase in the levels of gastric acid (P < 0.01, P < 0.05, respectively). Gastric prostaglandin E2 levels markedly increased in group 2 compared to group 1 (P < 0.01). Ileal prostaglandin E2 levels showed to be significantly higher in group 6 when compared with groups 2, 4, and 5 (P < 0.05). Gastric acidity increased at 3 and 9 days, decreased thereafter at 15 days following massive proximal SBR. While resected rats had increased levels of gastric prostaglandin E2 at 3 days, ileal prostaglandin E2 was markedly elevated at 15 days. Therefore, we conclude that prostaglandin E2 may have a possible role in regulating intestinal adaptation at the end of the adaptive process, and contribute to cytoprotective barrier function in the ileum and stomach at early and late periods of the intestinal adaptation, respectively.

Omentum pediculed musculo-peritoneal flap (OPMP) for growing intestinal neomucosa in a rat model.

BACKGROUND/PURPOSE: To construct a musculo-peritoneal flap adequately vascularised through attached omentum and to evaluate whether this flap is functional for growing new intestinal mucosa. METHODS: 24 rats underwent two surgical procedures. 1) A musculo-peritoneal flap was prepared, omentum was fixed onto the muscular side and a silicon graft was placed on the peritoneal side. 2) After one month the so constructed OPMP flap was mobilised and attached to the jejunum. Animals were harvested at 2, 4 and 8 months for macroscopic and microscopic evaluation. RESULTS: One rat died at 7 months due to intestinal volvulus. In the remainder the circumference of the patched intestine significantly increased compared to that of normal intestine (p<0.001). The flaps were markedly contracted in the 4 month rats (p<0.01). All flaps were completely covered by neomucosa except in the 2 month rats. Crypt depth and crypt density of the neomucosa was significantly lower in 2 month rats compared to other groups (p<0.05). Similarly neomucosal villous height was markedly low in the 2 month rats (p<0.01). CONCLUSIONS: To date, we have found no reports of small bowel patching using this technique. In this context, use of OPMP flap is likely to be useful for increasing intestinal surface area.

Multidirectional and simultaneous evaluation of gastroschisis-related intestinal damage in chick embryos.

PURPOSE: In a chick model of gastroschisis, we aimed to investigate the morphological/cellular, molecular, and ultrastructural changes taking place in gastroschisis-related intestinal damage (GRID). METHODS: 13-Day fertilized eggs were divided into two groups. CONTROL GROUP: chorio-amnio-allontoic membranes opened and abdominal wall exposed. Gastroschisis group: an anterior abdominal wall defect created after opening membranes. Embryos from both groups were surgically removed on post-fertilization day 19. Intestinal samples were obtained for histopathology, immunohistochemistry, molecular biology, and electron microscopy. RESULTS: The histopathological grade of intestinal damage which primarily involved mucosal structures was significantly higher in the gastroschisis group when compared to the control group (p<0.001). Immunohistochemically, E-cadherin and synaptophysin immunoreactivity in the gastroschisis group was significantly lower than control group (p<0.05 and p<0.01, respectively), whereas there was no significant difference in laminin and type-4 collagen immunoreactivity between the groups (p>0.05). Molecular analyses indicated a significant decrease in NFκB and IκB expression in the gastroschisis group (p<0.05 and p=0.001, respectively). Electron microscopy showed that the gastroschisis group had considerable ultrastructural damage, manifested by apoptosis in all layers. CONCLUSIONS: GRID affected all layers but was more prominent in mucosa. The damage may depend on E-cadherin and synaptophysin downregulation. Increased apoptotic activity, associated with decreased NFκB and IκB expression, may be an important component of this multifactorial damaging process.

Does noncomplicated acute appendicitis cause bacterial translocation?

It is unknown whether noncomplicated acute appendicitis cause bacterial translocation. In this study, we aimed to test development of the bacterial translocation in the patients who were operated for acute appendicitis. In this prospective study, 10 control patients who underwent elective operations because of other reasons, and 18 patients with noncomplicated acute appendicitis were evaluated. No patients took prophylactic antibiotic. After laparotomy, samples were obtained from peritoneal leaf just close to wound edge, and peritoneal swab culture from right paracolic region. Before appendectomy, a mesenteric lymph node (MLN) adjacent to the terminal ileum was taken out. Tissue samples were placed in a sterile container for microbiological analysis, and 10% formalin for histopathological analysis. Control samples had no bacterial translocation. Only 3 of 18 (16.6%) patients with appendicitis included bacterial translocation to MLN. There was no significant difference between both groups. No bacterial colonization was detected in the peritoneal tissue and peritoneal swab culture. Peritoneal tissue injury score was 2 +/- 1.4 in controls and 2.8 +/- 1.7 in the patients with appendicitis (P>0.05). MLN injury score was 2.5 +/- 1.3 in controls and 3.2 +/- 1.5 in the patients with appendicitis (P>0.05). No patient developed wound and systemic infection. No significant bacterial translocation frequency and tissue injury score was identified in the children with noncomplicated acute appendicitis. This result suggests that antibiotic prophylaxis may be unnecessary in such patients.

Midline dorsal plication to repair recurrent chordee at reoperation for hypospadias surgery complication.

PURPOSE: Midline dorsal plication is an efficient and safe surgical technique to correct chordee. We investigated the efficacy of midline dorsal plication for recurrent chordee in complicated hypospadias reoperations. MATERIALS AND METHODS: We retrospectively evaluated the charts of 25 boys who underwent reoperation between 1999 and 2004 due to complications of primary hypospadias repair other than meatal stenosis. A total of 15 cases were initially managed elsewhere for primary repair or complications. The etiology of recurrent chordee was defined at surgical correction. When recurrent chordee was noted a midline dorsal plication was performed. RESULTS: Of 25 patients 10 had previously undergone chordee repair. Nine of these patients were observed to have recurrent chordee and 1 had de novo chordee. A total of 10 patients had recurrent or delayed onset chordee. Mean patient age at primary repair was 6.28 years (range 1 to 33). Mean age at last operation for chordee was 15.9 years (range 4 to 66). Mean interval to recurrent chordee was 6 years (range 1 to 16), excluding a 66-year-old blind patient who did not know when recurrent chordee developed. Five patients had chordee recur before puberty at a mean interval of 2.6 years. Mean reoperation rate was 2.4 for recurrent chordee cases and 2.6 for chordee-free cases. Mean followup after midline dorsal plication for recurrent chordee repair was 22 months (range 8 to 56), while mean followup in pubertal and postpubertal cases was 20 months. No recurrence of chordee or surgery related morbidity was observed after recurrent chordee repair by midline dorsal plication. CONCLUSIONS: Chordee may recur during puberty following successful chordee repair. The midline dorsal plication technique is simple, efficient and safe even in patients who have undergone multiple surgeries for hypospadias and chordee repair.

Severe ischemia of the glans penis following circumcision: a successful treatment via pentoxifylline.

Severe ischemia or necrosis of glans penis is rare. We report the case of an 11-year-old boy with severe glanular ischemia occurring 24 h after circumcision. This was successfully treated with pentoxifylline injection for 5 days, and while the black color of the glans penis changed to brownish at 48 h, appearances were close to normal at 5 days. The patient did not require any surgical intervention, and was discharged without sequelae. We suggest that pentoxifylline might be considered as a treatment of choice for severe ischemia of glans penis.

Currarino syndrome associated with penoscrotal inversion and perineal fissure.

We present an unusual case of Currarino syndrome with a mucosa-lined deep perineal fissure extending to the sacrum, penoscrotal transposition, perineal hypospadias, and a penile ventral skin defect. The child had a sigmoid diverting colostomy because of high anal atresia. Magnetic resonance imaging illustrated absence of the levator ani and muscle complex in the pelvis. At 15 months, perianal examination pointed out a fistula orifice and a sac related to the fistula at the left side of the perineal fissure. The fistula, a fluid-filled sac extending to the sacrum, and mucosa overlying the perineal fissure were removed en bloc. The neck of the sac was ligated and divided at the level of the distal sacrum. In the same session, a Glenn-Anderson procedure was performed for penoscrotal transposition, and the penile chordee was released. X-ray showed a bony deformity of the sacrococcygeal region in the shape of a scimitar. Histopathological examination demonstrated that the sac contained glial neuronal islands and nerve fibers. The boy has no neurologic deficits and seems to be well. To our knowledge, these associated malformations are extremely rare.

Can alpha-blocker therapy be an alternative to biofeedback for dysfunctional voiding and urinary retention? A prospective study.

PURPOSE: Traditional treatment of dysfunctional voiding in children with urinary retention involves retraining the pelvic floor muscles using biofeedback. Alpha-blockers are reported to also be effective in children with urinary retention and dysfunctional voiding. We compared the efficacy of biofeedback and alpha-blockers for dysfunctional voiding and urinary retention in terms of residual urine volume and urge incontinence episodes, mean flow rates and urinary tract infections. MATERIALS AND METHODS: A total of 28 patients with a mean age of 6.25 years (range 4 to 10) presented with symptoms of urinary incontinence, urgency and urinary tract infections without anatomic and neurogenic causes of urinary retention. All patients had increased post-void residual (PVR) urine volume (mean 59 ml, 32% of age expected capacity [AEC]). The biofeedback group consisted of 16 children (mean age 6.5 years) and the alpha-blocker group consisted of 12 children (mean age 5.9 years). Both groups were also on continued timed voiding, constipation treatment and anticholinergics, which had been used for at least the last 6 months. Biofeedback (median 10, range 6 to 16 sessions) and doxazosin (0.5 to 2 mg) were administered. At 3 and 6 months incontinence episodes, urinary tract infections, mean urinary flow rates, PVR and parental satisfaction grades (1 to 10) were reevaluated. Six refractory cases were started on alpha-blockers and biofeedback, and reevaluated after 1 month and 3 months. RESULTS: Pretreatment mean PVR was 54 ml (30% of AEC), and mean posttreatment PVR was 21 ml (12% of AEC) and 9 ml (5% of AEC) at 3 and 6 months in the biofeedback group (p <0.05). Pretreatment mean PVR was 64 ml (38% of AEC), and posttreatment mean PVR was 17 ml (12% of AEC) and 13 ml (8% of AEC) at 3 and 6 months in the alpha-blocker group (p <0.05). There was no statistical difference in posttreatment PVR between the 2 groups (p >0.05). High PVR persisted in 4 (25%) biofeedback cases and in 2 (16%) alpha-blocker cases. Complete improvement in urge incontinence episodes occurred in 10 (62.5%) and 7 (70%) children in the biofeedback and alpha-blocker groups, respectively. In therapy responsive children parental satisfaction was higher with alpha-blocker than with biofeedback (9.2 vs 7.9, p <0.05). Refractory high PVR decreased significantly after combination treatment with biofeedback and alpha-blocker in 5 of 6 children (mean 80 ml, 35% of AEC vs mean 15 ml, 7% of AEC). No drug related side effect was reported in the alpha-blocker group. CONCLUSIONS: Alpha-blocker therapy seems to be a viable alternative to biofeedback in dysfunctional voiding in children with urinary retention to improve bladder emptying. Combination treatment (biofeedback and alpha-blockers) can be used as additional therapy in refractory cases.

The effect of location of the ureteric orifice on the efficacy of endoscopic injection to correct vesico-ureteric reflux.

OBJECTIVE: To review our 11-year experience and identify the mechanisms responsible for the failure of endoscopic injection for vesico-ureteric reflux (VUR) with three different injectable agents, based on the location of the ureteric orifice on endoscopy. PATIENTS AND METHODS: We retrospectively reviewed the charts and endoscopic video-photographs of 46 patients (26 girls, 20 boys, median age 6 years, range 2-16) with VUR treated once or twice by subureteric injection with PTFE, or polydimethylsiloxane or dextranomer/hyaluronic acid copolymer, from 1992 to 2003. Five patients were lost to follow-up and six ectopic and/or duplicated ureters were excluded from the analysis; in all, 52 ureters were analysed. According to the international classification, the VUR was grades I to V in four (8%), 12 (23%), 16 (31%), 13 (25%) and seven (13%) ureters, respectively. RESULTS: After 3 months, voiding cysto-urethrography showed that VUR continued in six of 19, seven of 12 and eight of 21 ureters (38%), respectively, after subureteric PTFE, polydimethylsiloxane and dextranomer/hyaluronic acid copolymer injection; after the second injection, reflux continued in two of six, four of seven and three of eight ureters, respectively. Mound displacement and/or volume loss was the most common failure with all three bulking agents after both the first (62%) and second injections (44%) (P < 0.05). The first injection failed in 32% (11 of 35) normally located ureters and 10 of 17 lateral ureters (P < 0.05). The second injection failed in 11% (four of 35) normal and five of 17 lateral ureters (P < 0.05). CONCLUSIONS: A lateral ureteric orifice may decrease the efficacy of endoscopic injection, as the likelihood of a faulty injection is greater. However, a more careful second injection decreases the failure rate, particularly in those with low- to medium-grade refluxing ureters.

A rare case of esophageal atresia with gastric outlet obstruction.

Although the combination of gastric outlet obstruction and esophageal atresia is rarely seen in neonates, it has been well described. We report the case of a 5-day-old newborn with esophageal atresia and tracheoesophageal fistula associated with complete gastric outlet obstruction due to a mucous plug. As the patient had intense gastric distention, severe respiratory distress requiring ventilatory therapy and complete pyloric obstruction in radiograms, emergency gastrostomy was performed before definitive operation. Definitive treatment consisted of tracheoesophageal fistula ligation and primary esophageal anastomosis. Exploratory laparotomy during the same session revealed a normal pyloric canal, completely obstructed by a firm mucous plug. The plug was removed by pylorotomy, and a pyloroplasty was performed to ease gastric evacuation. Postoperative feeding problems suggested gastric dysmotility as the possible cause for the mucous plug obstruction.

Sacrococcygeal teratoma showing organoid differentiation: report of a case.

We present the case of a neonate with a sacrococcygeal teratoma showing organoid differentiation. A 5-day-old baby girl was transferred to our hospital with a large sacrococcygeal mass. Ultrasonography revealed a few well-limited fluid-filled lesions, indicating that the teratoma had cystic components. During surgery, a sac containing 30cm of small bowel loops was found in the tumor. The bowel segment was supplied by a mesentery-like structure originating from teratoid tissue. Histopathological examination verified a benign mature teratoma with fully developed small bowel loops. The tumor was defined as a sacrococcygeal fetiform teratoma. The terminological, structural, and clinical aspects of this unusual tumor are discussed, with a review of the English literature.

Cryptococcal mesenteric lymphadenitis: an unusual cause of acute abdomen.

Cryptococcal infection of intraabdominal organs or tissues is extremely rare. Herein we report a child with mesenteric cryptococcal lymphadenitis who presented with an acute abdomen misdiagnosed as acute appendicitis. Definitive diagnosis was established with 2nd look and lymph node biopsy. Clinicians should remember that cryptococcal infection of mesenteric lymph nodes may rarely mimic an acute abdomen and cause delay in diagnosis.

Comparison of hypospadiac and normal preputial vascular anatomy.

PURPOSE: Data about the differences between the vascularization of normal and hypospadiac prepuce are lacking. We investigated the course of the preputial arterial blood vessels in normal controls and children with hypospadias by using transillumination, arterial methylene blue injection and 3-dimensional (3-D) reconstruction of serial histological sections focusing on arterial vessels. MATERIALS AND METHODS: Prepuce of 48 normal controls and 15 children with hypospadias was transilluminated by a front and back lighting technique and then photographed. All of the normal and 12 of hypospadiac prepuces not used for urethroplasty or penile body skin reconstruction were removed. The blood vessels of normal prepuce were also identified after arterial injection of methylene blue. Selected prepuce of normal controls and children with hypospadias was serially sectioned, and arterial and venous vessels were histologically distinguished. A 3-D computer reconstruction of the arterial system of normal and hypospadiac prepuces was performed. RESULTS: We confirmed the reliability of the transillumination technique to describe the arterial vascular anatomy of the prepuce by comparing the transillumination to methylene blue injection and 3-D reconstruction of histological sections. We classified the arterial vascular anatomy of normal prepuce as 1 artery predominant (41.67%), 2 arteries predominant (25%), H-type arching artery (12.5%) and net-like arterial system (20.83%). However, hypospadiac prepuce revealed a net-like arterial system more frequently (50%). We noted that the frequency of net-like arterial system was higher in more severe hypospadiac prepuce. CONCLUSIONS: Understanding the differences between normal and hypospadiac prepuce vascular anatomy is germane to hypospadias surgery. The arterial blood supply of the hypospadiac prepuce is different than normal. A better knowledge of the vascular anatomy of the hypospadiac prepuce may improve the surgical results of hypospadias repair.

The effects of pentoxifylline on renal function and free radical production in unilateral ureteral obstruction.

There is an ongoing discussion about ureteral obstruction-related renal dysfunction. In this study, we aimed to test the effect of pentoxifylline (PTX) on both kidneys in unilateral ureteral obstruction (UUO), and to determine its interaction of with prostaglandin E2 (PGE2), and diclofenac sodium (DIS). A sham operation was performed in group 1. Placebo, PTX, DIS, and PTX+DIS were administrated to groups 2, 3, 4 and 5, respectively. The left ureter was ligated in all groups except group 1. At 24 h, technetium 99m diethylenetriamine penta-acetic acid scintigraphy was performed to determine renal function. Additionally, the tissue levels of thiobarbituric acid reactive substances (TBARS) and PGE2 in both kidneys were measured to determine cytotoxic and cytoprotective mechanisms. When the ipsilateral kidneys were evaluated: (1) UUO significantly reduced DTPA uptake and none of the medications used prevented the reduction, (2) UUO significantly increased TBARS production, and only PTX prevented the increase, (3) UUO caused a significant increase in PGE2 production, and only DIS significantly decreased this. When the contralateral kidneys were evaluated: (1) UUO significantly increased DTPA uptake but DIS and PTX+DIS prevented this, (2) UUO significantly elevated TBARS levels and DIS and PTX+DIS caused an additional elevation, (3) UUO significantly increased PGE2 production, and only DIS prevented this. In conclusion, UUO caused ipsilateral renal hypofunction and contralateral hyperfunction, which are related to increased TBARS and PGE2 levels. PTX markedly decreased free radical activity in the ipsilateral kidney. While PTX showed a placebo effect, DIS prevented the compensatory contralateral renal response through increased TBARS and decreased PGE2 levels. The beneficial effect of PTX on the ipsilateral kidney, and the hazardous effect of DIS on the contralateral kidney may be explained by more complex interactions among TBARS, PGE2, PTX, DIS and UUO-related renal dysfunction.

Two rare cases of the Pentalogy of Cantrell or its variants.

The Pentalogy of Cantrell (PC) is a rare association of defects involving the lower sternum, abdominal wall, diaphragm, pericardium and heart. We report two rare cases of the PC (variant form), showing fatal progression. Case 1 only survived two hours because of severe cardio-respiratory failure. Physical examination showed midline abdominal and thoracic defects, ectopic heart, pericardial defect, diaphragmatic defect, bilateral undescended testis, scoliosis, and adherence between left upper limb and trunk. In addition, the autopsy revealed diaphragmatic agenesia, intraabdominal testis, bilateral lung hypoplasia and lymphocytic meningitis. Case 2 only survived 15 minutes. In addition to the physical findings, including lower sternal defect, ectopic heart, epigastric omphalocele and scoliosis, the autopsy showed left diaphragmatic agenesia, pericardial agenesia, bilateral lung hypoplasia, deformed rib cage, anterior thoracic myeloschisis, adreno-hepatic fusion, left renal agenesia, meckel diverticulum and multiple accessory spleens. When comparing with other cases of PC, the concurrence of bilateral intraabdominal testis and lymphocytic meningitis in case 1, and adreno-hepatic fusion, anterior myeloschisis, meckel diverticulum, multiple accessory spleens, and renal agenesia in case 2 have not been described previously.